Importance of botulinum toxin in symptomatic treatment of Wilson's disease

Toxicon ◽  
2008 ◽  
Vol 51 ◽  
pp. 26
Author(s):  
Damásio Joan ◽  
Cardoso Márcio Neves ◽  
Magalhães Marina
Toxins ◽  
2021 ◽  
Vol 13 (4) ◽  
pp. 241
Author(s):  
Harald Hefter ◽  
Sara Samadzadeh

Background: Recent cell-based and animal experiments have demonstrated an effective reduction in botulinum neurotoxin A (BoNT/A) by copper. Aim: We aimed to analyze whether the successful symptomatic BoNT/A treatment of patients with Wilson’s disease (WD) corresponds with unusually high doses per session. Methods: Among the 156 WD patients regularly seen at the outpatient department of the university hospital in Düsseldorf (Germany), only 6 patients had been treated with BoNT/A during the past 5 years. The laboratory findings, indications for BoNT treatment, preparations, and doses per session were extracted retrospectively from the charts. These parameters were compared with those of 13 other patients described in the literature. Results: BoNT/A injection therapy is a rare (<4%) symptomatic treatment in WD, only necessary in exceptional cases, and is often applied only transiently. In those cases for which dose information was available, the dose per session and indication appear to be within usual limits. Conclusion: Despite the evidence that copper can interfere with the botulinum toxin in preclinical models, patients with WD do not require higher doses of the toxin than other patients with dystonia.


2012 ◽  
Vol 70 (6) ◽  
pp. 407-409 ◽  
Author(s):  
Hélio A.G. Teive ◽  
Leandro E. Klüppel ◽  
Renato P. Munhoz ◽  
Nílson Becker ◽  
Paulo R. Müller ◽  
...  

We have reported a case series of five patients with jaw-opening oromandibular dystonia secondary to Wilson's disease (WD), in which the patients were treated with botulinum toxin type A (BTX-A). In all cases, dystonia score was partially reduced three weeks after injections. The most common side effect was transient mild dysphagia. This preliminary study showed that jaw-opening oromandibular dystonia in WD may be partially responsive to the use of BTX-A.


2018 ◽  
Vol 40 (2) ◽  
pp. 54-60
Author(s):  
Y. S. Tsimmerman

The article presents a detailed review of modern ideas on Wilson’s disease – hepatocerebral dystrophy. The definition, terminology, history of the study of the disease are stated. Special attention is paid to the analysis of the pathogenesis of hepatocerebral dystrophy, including the genetic basis of its development, the disturbance of copper metabolism. The clinical picture is thoroughly described, taking into account the characteristics of liver lesion and extrahepatic manifestations, diagnostics, classification. Particular attention is paid to the means of pathogenetic and symptomatic treatment.


2000 ◽  
Vol 37 (2) ◽  
pp. 187-189 ◽  
Author(s):  
P Luca ◽  
L Demelia ◽  
S Lecca ◽  
R Ambu ◽  
G Faa

2009 ◽  
Vol 40 (01) ◽  
Author(s):  
P Günther ◽  
W Hermann ◽  
A Wagner

2015 ◽  
Vol 53 (05) ◽  
Author(s):  
D Németh ◽  
A Folhoffer ◽  
A Krolopp ◽  
S László ◽  
Z Gerlei ◽  
...  

2006 ◽  
Vol 37 (01) ◽  
Author(s):  
W Hermann ◽  
T Villmann ◽  
HJ Kühn ◽  
P Baum ◽  
G Reichel ◽  
...  

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